6 -Relapses of short duration have been noted after complete clearing -Common 9months to 6 years but can occur earlier or later-Last 1-3 years-Relapses of short duration have been noted after complete clearing
7 Nevus DepigmentosisGenerally present at birth or within several months, sometimes not obvious until sun exposure in fairer skin Borders can be regular or irregular Can have whorled appearance
8 Pigmentary Mosacism /Hypomelanosis of ito Follows blashkos linesCan be associated with neurologic, skeletal, and/or ocular abnormalitiesThe hypopigmentation is secondary to hypofunctioning of melanocytes and is thought that mishap ear in embryogenesis is responsible for the maosaicism.No consistent genetic defect.Follow up depends on associated abnormalities.
9 Follows blashkos lines Can be associated with neurologic, skeletal, and/or ocular abnormalitiesThe hypopigmentation is secondary to hypofunctioning of melanocytes and is thought that mishap ear in embryogenesis is responsible for the mosaicism.No consistent genetic defect.Follow up depends on associated abnormalities.
10 Beckers NevusLarge dermal nevi commonly shoulder but not exclusivelyLarge, pigmented, hairy nevus Most commonly noted in adolescence Benign
12 Melan0nychia Striata Longitudinal Melanonychia Most commonly seen in individuals with darker skin- especially African-Americans in whom up to 90% may have at least one such streakThe pigmentation extends from the proximal nail fold to the distal margin of the digitThe width may vary from less than 1mm to several mmBut……. (there is always a but)
13 Nail Matrix Melanoma should be considered Worrisome features may include very dark, broad bands AND Extension of the pigmentation onto the proximal or lateral nail folds (aka Hutchinson’s Sign) Any patient with this presentation should be referred for nail matrix biopsy which can cause permanent damage to the nail plate
14 Multiple presentation is less worrisome for me but should check each individual nail for invasion nail folds
18 First described by Dr. Duncan in Houston in 1987 At fist glance these patients may appear to have confluent and reticulated papillomatosis (CARP) or acanthosis nigricansIn contrast to derma neglecta soap and water will not wash this off, isopropyl alcohol is necessaryWhen this condition is suspected, firm, persistent pressure should be applied while rubbing the skin with alcohol.The cause of terra firma-forme dermatosis is unknown.Noted at time of doing biopsy and area cleared with alcohol prep
20 If you mom think it is fungal….. This a variant of dry skinIt is usually asymptomaticAppears worse in summer, better during school yearTreatment is sunscreen and moisturizerIf you mom think it is fungal…..Do a culture
22 Very common!!Become more extensive in drier climate.Individual lesions represent plugs of stratum corneum in individual follicular openingsie: Skin TrashMoisturizer with urea of lactic, salycylic acidMay help but is not curative.
23 ID Reaction Hypopigmented thickened papules often on elbows , knees Autosensitization DermatitisautoeczematizationHypopigmented thickened papules often on elbows , kneesCan be eczematous and very itchy
24 -found in up to 50% of patients with nickel dermatitis -nearly always symmetrical-may also be seen in response to infectious agents, particularly in bacterial and dermatophyte infections.In the case of tinea capitis the id reaction is usually seen on the head and neck and is often after initial of oral antifungal agent and is erroneously diagnosed as drug reactionClearance occurs with resolving of the originial cause- nickel avoidance, clearance of tinea, etc.
26 Knuckle PADSKnuckle pads were medically first described by Garrod in 1893 and are also named Garrod's pads
27 There is no known established, generally successful therapy. Injection of corticosteroids (triamcinolone) can soften and sometimes even shrink the pads. Radiation therapy has also been reported to be successful in some cases but not consistently.Knuckle pads can be surgically removed in the case of pain associated with them. ..
28 Developmental defect rather than birthmark Aplasia Cutis CongenitaDevelopmental defect rather than birthmarkOccurs in about 1 in 5000 birthsUlcerated defects may heal with scar.
29 Although usually benign, the hair collar sign may be associated with other physical anomalies and malformation syndromes.No specific laboratory tests are required, although a hair collar sign signals the possibility of a CNS malformation and thus may warrant an MRI scan to rule out an underlying pathology
30 Nevus SebaceousAppears at birth as slightly raised yellow orange nevusAt puberty become raised and wartyBasal cell carcinoma and other benign tumors occur in 15-50% of the tumorsExcision recommended prior to puberty.
32 Urticaria Pigmentosa Hereditary Form Seen in less than 2% of children Often onset is after 12 months of age More lesions accumulate slowly with age and lesions do not spontaneously resolve. System symptoms develop with time in 2% of the children with this form (flushing, tachycardia, hypotension, hepatosplenomegaly
33 MastocytomaSolitary lesion Will not develop more Topical steroids for treatment of symptoms
34 Non Hereditary Form of Mastocytosis is much more common Blotchy macular and nodular pigmented lesions which appear in the first 8 months of life starts with one or 2 and then develop numerous lesions over months.The hyperpigmented appearance may not appear until 6 months after onsetDarriers sign- stroking the lesion will cause tense edema and rednessSpontaneous clearing by 6 or 7 years of age is usual
35 Essential that children with any type of Mastocytosis not be given the following medications: Opiates (codeine, demerol, morphine)Polymixin BAcetylsalicylic AcidIn the case of surgery very important that perioperative medications carefullySelected.
36 Treatment optionsNonsedating Antihistamine (zyrtec, Allegra, etc) Regular daily dosing Can try every 6 months to stop and if starts flaring then resume medication Topical Steroids for itching in individual lesions BID For body Triamcinolone 0.025% or 0.1% For face/neck/groin/axilla/head Hydrocortisone 2.5% or Desonide 0.05%.
40 Propranolol Protocol at CMC Dermatology Prior to initiation of therapy needs : BP Pulse Chest Xray ECG Propranolol 1mg/kg/dose administer BID (20mg/5ml solution)
41 PropranololPropranolol 1mg/kg/dose administer BID 20mg/5ml solution Taper Schedule: Days mg/kg/dose BID Days mg/kg/dose BID Days mg/kg/dose BID Day 14 and on 1mg/kg/dose BID Can also divide the above doses to TID (.025mg/kg x2) 3
42 Follow up ScheduleFollow up is weekly x 3,then q2w x 4, then montly. BP q visit. Infants younger than 3 months should have CBG monitored each visit. Parents should be instructed how to count pulse and recognize low heart rate They should watch for somnolence, perioral pallor, cold hands and feet. Patients must be fed q4h while on this medication due to hypoglycemia possibility
53 Dermatology information resources Textbooks: Color Textbook of Pediatric Dermatology- Weston, Lane, and Morelli Pediatric Dermatology- Bernard A Cohen Great for pictures and information that is clinically helpful. DermNet NZ. Facts about the skin. Skin Advocate App- available for free in app store- has information on parent and patient support organizations and is set up for you to the address directly to the patient with a share line. (Developed by UTSW Resident and received national award !)
54 Some things can be fixed and some cannot….. However we do know that education and informationis just as valuable as cures can be.The goal is always………….